Zhang Hanyu, Yan Dingfang, Wei Yuehua, He Yun, Chang Junjie, Zhang Wenjun
Department of Medical Ultrasound, Taihe Hospital, Hubei University of Medicine, Shiyan, China.
Cancer Center, Renmin Hospital of Wuhan University, Wuhan, China.
Front Cardiovasc Med. 2024 Jul 18;11:1434734. doi: 10.3389/fcvm.2024.1434734. eCollection 2024.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of systemic diseases caused by a combination of many factors, including genetics, environment, and immunity. AAV is characterized by predominantly small-vessel involvement and has a variety of clinical manifestations. Small-vessel lesions of the kidneys and lungs are common, and lesions of medium-sized arteries may also present, but the involvement of large arteries and their primary branches is very rare. This report delineates two instances of AAV with large arterial involvement, one case presenting with lesions of the aortic valve and the other with lesions of the pulmonary artery. The first case involved a 57-year-old man with no underlying diseases. Transthoracic echocardiography showed thickening of the left and right coronary valves of the aortic valve with enhanced echogenicity, moderate echogenic masses were seen on both valve leaflets, and the leaflets had restricted opening and poor closure. Blood tests showed positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) and anti-myeloperoxidase (MPO) antibodies. The patient's aortic valve thickening virtually disappeared after treatment with hormones combined with immunosuppressive agents. The second case involved a 60-year-old woman whose transthoracic echocardiography and CT (computed tomography) angiography of the pulmonary arteries showed wall thickening of the main pulmonary artery and the proximal left and right pulmonary arteries, leading to luminal stenosis. Blood tests showed positive cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and anti-proteinase 3 (PR 3) antibodies. The patient's pulmonary artery wall thickening reduced after receiving hormones in combination with immunosuppression but she died of heart failure during subsequent treatment. The patient had been diagnosed with tuberculosis six months earlier and had been poorly treated with anti-tuberculosis therapy. The involvement of large arteries in AAV is a rare and critical condition with rapid progression and a high mortality rate. Early recognition of this type of AAV and aggressive immunosuppressive therapy may facilitate the reversal of the vascular lesion and a reduction in the risk of patient death.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一组由多种因素共同作用引起的系统性疾病，这些因素包括遗传、环境和免疫等。AAV的特征主要是小血管受累，具有多种临床表现。肾脏和肺部的小血管病变较为常见，中等大小动脉的病变也可能出现，但大动脉及其主要分支受累非常罕见。本报告描述了两例伴有大动脉受累的AAV病例，一例表现为主动脉瓣病变，另一例表现为肺动脉病变。第一例患者为一名57岁无基础疾病的男性。经胸超声心动图显示主动脉瓣左右冠状动脉瓣增厚，回声增强，两个瓣膜小叶均可见中等回声团块，小叶开口受限且关闭不佳。血液检查显示核周型抗中性粒细胞胞浆抗体（p-ANCA）和抗髓过氧化物酶（MPO）抗体阳性。经激素联合免疫抑制剂治疗后，患者的主动脉瓣增厚几乎消失。第二例患者为一名60岁女性，其经胸超声心动图和肺动脉CT（计算机断层扫描）血管造影显示主肺动脉及左、右肺动脉近端壁增厚，导致管腔狭窄。血液检查显示胞浆型抗中性粒细胞胞浆抗体（c-ANCA）和抗蛋白酶3（PR 3）抗体阳性。患者接受激素联合免疫抑制治疗后肺动脉壁增厚减轻，但在后续治疗中死于心力衰竭。该患者6个月前被诊断为肺结核，抗结核治疗效果不佳。AAV累及大动脉是一种罕见且严重的疾病，进展迅速，死亡率高。早期识别此类AAV并积极进行免疫抑制治疗可能有助于逆转血管病变并降低患者死亡风险。
